[Secretory carcinoma of salivary gland: a clinicopathological and prognostic analysis of twelve cases]

Zhonghua Bing Li Xue Za Zhi. 2021 Aug 8;50(8):899-903. doi: 10.3760/cma.j.cn112151-20210327-00238.
[Article in Chinese]

Abstract

Objective: To study the clinical manifestations, pathological features, molecular features, differential diagnosis and prognosis of secretory carcinoma of salivary gland (SCSG). Methods: Twelve cases of SCSG diagnosed in the Department of Pathology, the First Affiliated Hospital of Zhengzhou University from January 2017 to December 2019 were collected and analyzed in terms of histological morphology, immunohistochemistry (Envision method) and molecular detection. Results: Among the 12 patients, there were 6 males and 6 females, aged 12-67 years old, with a median age of 41.5 years. The lesions in 11 patients were located in the parotid gland. The maximum diameter of the tumors ranged from 0.8 to 5.5 cm. Clinically, slow-growing painless or tender mass was the first symptom, and all patients underwent surgical resection, among which 5 patients received postoperative radiotherapy and chemotherapy, and 1 patient developed local recurrence. Histopathologically, the tumor is characterized by microcystic, solid or tubular structures with unique intravitary homogeneous secretions. Immunohistochemistry showed diffuse expression of CK7, mammaglobin, GATA-3 and S-100 in all cases, and pan-Trk in 10 of the 12 cases. Sox-10 was expressed focally in 9 cases, and Ki-67 index was 5%-20%. In molecular detection, 11 cases had ETV6 gene break/fusion. Conclusions: SCSG is a relatively rare low-grade malignant salivary gland tumor, with typical histological morphology and immunophenotype, Pan-Trk immunohistochemistry may be related to NTRK fusion, ETV6-NTRK3 gene rearrangement is not only of diagnostic significance, but also Trk-targeted therapy is expected to play a greater role in clinical treatment.

目的: 探讨涎腺分泌性癌(secretory carcinoma of salivary gland,SCSG)的临床表现、病理学特征、分子特征、鉴别诊断及预后。 方法: 收集2017年1月至2019年12月郑州大学第一附属医院病理科确诊的12例SCSG,从组织学形态、免疫组织化学(EnVision法)及分子检测多方面研究分析。 结果: 12例SCSG患者男女各6例,年龄12~67岁,中位年龄41.5岁,11例病变位于腮腺,肿瘤直径0.8~5.5 cm,临床上均以缓慢生长的无痛或压痛性肿块为首发症状,所有患者均行手术切除,其中5例接受了术后放化疗,1例出现局部复发。病理组织学上,肿瘤以微囊状、实性或管状结构为主,伴有独特腔内均质分泌物。免疫组织化学上,12例均弥漫表达细胞角蛋白(CK)7、乳腺球蛋白、GATA-3和S-100蛋白,10例表达pan-Trk,9例灶性表达SOX-10,Ki-67阳性指数5%~20%。分子检测上,有11例存在ETV6基因断裂/融合。 结论: SCSG是一种较为罕见的低度恶性涎腺肿瘤,具有典型组织学形态和免疫表型,pan-Trk免疫组织化学可能与NTRK融合相关,ETV6-NTRK3基因重排不仅有诊断意义,同时Trk靶向治疗也有望在临床治疗中发挥更大的作用。.

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Biomarkers, Tumor / genetics
  • Carcinoma*
  • Child
  • Female
  • Humans
  • Male
  • Middle Aged
  • Oncogene Proteins, Fusion / genetics
  • Prognosis
  • Salivary Gland Neoplasms* / genetics
  • Salivary Glands
  • Young Adult

Substances

  • Biomarkers, Tumor
  • Oncogene Proteins, Fusion